Index of drugs

Prophylaxis and treatment of bleeding in patients with: haemophilia B (congenital deficiency of IX coagulation factor).

1 vial contains 300 IU, 600 IU or 1200 IU, respectively. human IX blood coagulation factor.

Hypersensitivity to the active substance or to any of the excipients.

Due to the rare occurrence of haemophilia type B in women, data on the use of factor IX during pregnancy and during breastfeeding are not available. Therefore, the administration of the preparation during pregnancy and lactation should be highly justified.

Disorders of the immune system. Hypersensitivity or allergic reactions (including angioedema, pricking and burning at the injection site, chills, flushing and headache, generalized urticaria, headache, urticaria, hypotension, lethargy, nausea, anxiety, tachycardia , chest tightness, tingling sensation, vomiting, wheezing) have been very rarely seen in patients treated with formulas containing IX factor. In very rare cases, these symptoms developed severe anaphylaxis and were associated temporarily with the formation of factor IX inhibitors. Haemophilia B patients may very rarely produce neutralizing antibodies (inhibitors) of IX coagulation factor. If these types of inhibitors appear, this condition will manifest itself as an insufficient response to treatment. In such cases, a specialized haemophilia center should be contacted. The experience in the use of the preparation in previously untreated patients, obtained on the basis of clinical trials, is very limited. Therefore, data on the incidence of specific inhibitors of clinical relevance are not available.General disorders. In very rare cases, elevated body temperature has been observed.Renal and urinary disorders. There was one case describing the occurrence of nephrotic syndrome as a consequence of attempts to induce immune tolerance in patients with haemophilia B who had factor IX inhibitors and a history of allergic reactions.Vascular disorders. In very rare cases, there is a potential risk of thromboembolic complications when using preparations containing factor IX. It is increased when using low-purity products. The use of low-purity products is associated with the occurrence of myocardial infarction, intravascular coagulation, venous thrombosis and pulmonary embolism. The use of highly purified IX products is rarely associated with such disorders.Blood and lymphatic system disorders. Very rare cases have been reported in patients with type II heparin-dependent thrombocytopenia (HAT Type II), when the platelet count drops significantly below 100,000 per μl or is less than 50% of their initial value. In patients who have not previously had hypersensitivity to Heparin, a fall in the platelet count usually occurs 6 to 14 days after the start of treatment. In patients hypersensitive to heparin, such a decrease may occur within a few hours of its administration. The severe form of thrombocytopenia may be due to blood clots / thromboembolism in the arteries or veins, consumption coagulopathy, which in some cases may be accompanied by necrosis of the skin at the injection site, ecchymosis, purpura and black stool. In such cases, the heparin-inhibiting effect on the coagulation process may be reduced (heparin tolerance). In patients who have experienced these allergic reactions, the administration should be discontinued immediately. Such patients should be informed that they are not allowed to take any heparin-containing medicines in the future.

Treatment of haemophilia should be under the supervision of a physician experienced in the treatment of haemophilia.Dosage and duration of substitution therapy depends on the severity of the factor IX deficiency of blood clotting factor, location and intensity of bleeding, as well as on the clinical condition of the patient. The number of units of the coagulated factor IX administered is expressed in International Units (IU), which refer to current WHO standards for coagulation factor IX products. Plasma factor IX activity is expressed as a percentage (relative to human plasma) or in IU. (with reference to international standards for the content of IX blood clotting factor in plasma). 1 international unit (IU) of factor IX activity is equal to the amount of IX coagulation factor contained in 1 ml of normal human plasma. The conversion of the required dose of factor IX of the coagulation factor is based on the empirical finding that 1 IU. IX factor of coagulation per kilogram of body mass increases the activity of factor IX in plasma by 1% of normal activity. The calculation of the required dose is made using the following formula: required number of units = body weight [kg] x desired increase in factor IX activity [%] (IU / dl) x {inverse of the observed improvement}. The dose of the preparation and the frequency of its administration and duration of therapy should always be individually selected depending on the clinical effectiveness of individual patients. IX blood coagulation factor rarely requires administration more than once a day. If the following bleeding events occur, factor IX activity should not fall below the reported plasma activity (in% of standard or IU / dL) for the corresponding time period. Here are some tips for bleeding and surgery.Bleeding. A minor haemarthrosis or muscle bleeding in the mouth - therapeutic level of factor IX in plasma (%) (IU / dL) - 20-40 - repeat the infusion every 24 hours for at least 1 day until the pain disappears and cessation of blood or cure. Extensive haemorrhage, muscle or hematoma - therapeutic level of plasma factor IX activity (%) (IU / dL) - 30-60 - repeat infusion every 24 h for 3-4 days or longer until the pain or acute organ dysfunction disappears . Life threatening haemorrhage - therapeutic level of factor IX activity in plasma (%) (IU / dl) - 60-100 - repeat the infusion every 8-24 h until the hazard disappears.Surgical procedures. Minor treatments including tooth extraction - therapeutic level of factor IX activity in plasma (%) (IU / dl) - 30-60 - every 24 h, for at least 1 day, until the symptoms disappear. Significant surgical procedures - therapeutic level of factor IX in plasma (%) (IU / dL) - 80-100 (before and after surgery) - repeat the infusion every 8-24 h, until the expected healing of the wound, then for at least 7 days until to obtain from 30% to 60% (IU / dL) of the coagulation factor IX activity. During treatment, it is recommended to appropriately measure the level of factor IX in order to determine the dose to be administered and the frequency of repetition of infusions. During major surgical procedures, it is necessary to precisely monitor the substitution therapy by controlling the coagulation process (the level of activity of the coagulation factor IX in the plasma). Individual patients may have differences in the response to IX administration of coagulation factor, which reflects the differences in recoveryin vivo and in the half-life. In long-term prophylaxis of bleeding in patients with severe haemophilia B, doses of factor IX are usually 20 to 40 IU. per kg bw at intervals of 3 to 4 days. In some cases, especially in younger patients, higher doses or shorter intervals between doses may be necessary. Patients should be monitored for possible coagulation factor IX inhibitors. If the expected level of factor IX activity in the plasma is not reached or bleeding is not controlled by an appropriately calculated dose, a test for coagulation factor IX inhibitors should be performed. In patients with high levels of inhibitor, treatment with coagulation factor IX may be ineffective, so other therapeutic measures should be considered. Treatment of these patients should be carried out by a doctor who has experience in the care of patients with haemophilia. There are no data from clinical trials to determine the dosage of the preparation in children.
The reconstituted solution should be warmed up to room temperature or body temperature before administration. Administer slowly intravenously using injection or infusion at a rate convenient for the patient.Ensure that blood does not get into the syringe with the product. The rate of injections or infusions should not exceed 2 ml / min. The patient should be observed in the event of an immediate reaction. If any patient reactions occur that may be associated with the administration of the preparation, the infusion should be discontinued or its speed limited, depending on the clinical condition of the patient.